Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula

Artery Fistula Causing Aortic Regurgitation in Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

“…Occasionally, pulmonary blood flow may arise from a coronary artery-to-pulmonary artery (CAPA) fistula. Previous related studies have not reported any cases of coronary steal, myocardial ischemia, or aortic regurgitation (AR) resulting from a CAPA fistula [1]. Our patient experienced a rare case of AR and CAPA fistula with PA/VSD and MAPCAs.…”

“…In our patient, the fistula arose from the left anterior descending branch. Although CAPA fistulas steal coronary artery blood flow, there have been no reported cases of myocardial ischemia [1,2]. Coronary artery perfusion may be independent of the pulmonary bed and dependent only on the distal coronary bed as long as the proximal coronary artery is patent without stenosis and flow limitation [2,3].…”

“…Our approach to the management of PA/VSD with MAPCAs focused on early operative intervention to prevent the pulmonary vascular bed from obstructing changes or to improve preservation of heart function and control for heart failure after the operation [1]. This strategy may increase the success of staged operative management.…”

Management of Pulmonary Arterial Supply Dependent on a Coronary Arterial Fistula in a Patient With Tetralogy of Fallot With Pulmonary Atresia

“…This hypothesis is endorsed by the finding that, despite a significant runoff of the blood from the proximal coronary artery into the pulmonary arteries, so far no case has been reported with features of either coronary ischemia or insufficiency. [3][4][5] Also, in these patients, the size of the communication between the coronary artery and the pulmonary arteries is restricted. Hence, there is only mild-to-moderate elevation of pulmonary arterial pressure.…”

“…1,2 Sometimes the arteries can take their origin from the coronary arteries. [3][4][5] Much more rarely, a fistula from the coronary arteries to the pulmonary arterial tree may be the predominant source of pulmonary supply. 6 We describe here the surgical management of a patient with TOF and PA, in whom a fistula from the anterior interventricular coronary artery (LAD) was the predominant source of arterial supply to both the lungs.…”